title: The epigenetics of cancer in children.
abstract: Malignant tumors of childhood represent a rather heterogeneous group of neoplasms originating from virtually any anatomical structure. Despite major improvements in the clinical management including timely diagnosis, advanced supportive care and refined multimodality treatment, prognosis remains grim for certain risk groups. Aberrant epigenetic regulation, i.e. changes in gene transcription not due to DNA sequence alterations, is now increasingly recognized as a fundamental process in malignant transformation, tumor progression and drug resistance. The molecular mechanisms involve aberrant activity of enzymes controlling the packaging and transcriptional regulation of the genome. Two major protein families are involved in this process, DNA methyltransferases and histone deacetylases. With the availability of small molecule inhibitors targeting the aberrant epigenetic machinery in cancer cells, these compounds are evaluated in several clinical trials.
authors.affiliation: Department of Pediatric Hematology and Oncology, University Children's Hospital Münster, Germany. Michael.Fruehwald@ukmuenster.de
authors.forename: M C
authors.initials: MC
authors.lastname: Frühwald
authors.forename: O
authors.initials: O
authors.lastname: Witt
mesh_terms: D000818:Animals; D000970:Antineoplastic Agents / Q000009:adverse effects / Q000627:therapeutic use; D002471:Cell Transformation, Neoplastic / Q000235:genetics; D002648:Child; D003562:Cytidine / Q000009:adverse effects / Q000031:analogs & derivatives / Q000627:therapeutic use; D015254:DNA Modification Methylases / Q000037:antagonists & inhibitors / Q000235:genetics*; D018450:Disease Progression; D019008:Drug Resistance, Neoplasm; D015507:Drugs, Investigational / Q000009:adverse effects / Q000627:therapeutic use; D044127:Epigenesis, Genetic / Q000235:genetics*; D056572:Histone Deacetylase Inhibitors; D006655:Histone Deacetylases / Q000235:genetics; D006801:Humans; D009369:Neoplasms / Q000188:drug therapy / Q000235:genetics*; D014158:Transcription, Genetic / Q000235:genetics
chemical_list: D000970:Antineoplastic Agents; D015507:Drugs, Investigational; D056572:Histone Deacetylase Inhibitors; D003562:Cytidine; C009131:pyrimidin-2-one beta-ribofuranoside; D015254:DNA Modification Methylases; D006655:Histone Deacetylases